Department of Surgery, RuijinHospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Pancreas. 2010 Jul;39(5):680-3. doi: 10.1097/MPA.0b013e3181d36341.
Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disorder characterized by the development of parathyroid hyperplasia, pancreatic endocrine tumors, pituitary adenomas, and adrenal adenomas. We reported 1 case of MEN 1 simultaneous with gastrinoma and insulinoma; meanwhile, insulinomas were ectopic and recurrent. The genetic screening showed the mutation of 427del AT of the MEN 1 gene. Surgical removal is considered the treatment of choice, with limited adverse effects and relatively low morbidity and mortality. She was treated by means of several surgical strategies, resulting in improvement of the frequency and severity of the hypoglycemic episodes and a better quality of life.
多发性内分泌腺瘤病 1 型（MEN1）是一种常染色体显性遗传疾病，其特征是甲状旁腺增生、胰腺内分泌肿瘤、垂体腺瘤和肾上腺腺瘤的发展。我们报告了 1 例 MEN1 同时伴有胃泌素瘤和胰岛素瘤的病例；同时，胰岛素瘤为异位且复发性的。基因筛查显示 MEN1 基因 427delAT 的突变。手术切除被认为是治疗的首选方法，具有有限的不良反应，且发病率和死亡率相对较低。她采用了多种手术策略进行治疗，结果改善了低血糖发作的频率和严重程度，提高了生活质量。
